Kidney - neoplasia Flashcards
What is an angiomyolipoma?
hamartoma composed of Bvs, SM, adipose tissue
What is the symptomatic triad in renal cell carcinoma?
hematuria, palpable mass, flank pain
Who is the typical person to get RCC?
male smoker in his 60s
What are some paraneoplastic syndromes typically involved in renal cell carcinoma?
inc EPO
inc renin
PTHrP
ACTH
What molecularly occurs in renal cell carcinoma?
loss of VHL - inc IGF1, inc HIF
If a person has VHL disease, what are they at risk for?
hemangioblastoma of the cerebellum, retinal angiomas, pheochromocytoma
Where does renal cell carcinoma like to spread to?
lungs and bones
What is a wilms tumor composed of?
blastema, primitive glomeruli, tubules, stromal cells
What mutation are wilms tumors associated with?
WT1
What are three syndromes associated with WT1 mutation?
- WAGR syndrome
- beckwith wiedemann syndrome
- denys drash
What are the findings in WAGR syndrome?
W - wilms
A - aniridia
G - genital anomalies
R - mental retardation
What are the findings in beckwith wiedemann syndrome?
- wilms
- neonatal hypoglycemia
- muscular hemihypertrophy
- organomegaly - especially the tongue
What are the findings in denys drash syndrome?
- wilms
- intersexual disorders
- glomerular mesangial sclerosis
What are some risk factors for urothelial carcinoma?
cig smoke - polycyclic hydrocarcbons, napthylamine
azo dyes
cyclophosphamide, phenacetin
How does urothelial carcinoma usually present?
painless hematuria in elderly people
